Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease Evidence for a disease specific abnormality of the myocardial interstitium

Moon, James; Sachdev, Bhavesh; Elkington, Andrew G.; McKenna, William J.; Mehta, Atul; Pennell, Dudley J.; Leed, Philip J; Elliott, Perry

doi:10.1016/j.ehj.2003.09.017

Cited by 415 publications

(291 citation statements)

References 21 publications

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“…Like described for other cardiomyopathies, CMR is the imaging technique of choice in suspected cardiac AFD involvement. LGE within the basal infero-lateral wall without affecting the endocardium is characteristic for a cardiac manifestation in AFD [68]. In genetically confirmed AFD, Moon et al demonstrated that LGE of the infero-lateral wall was present in 50 % of the patients [68].…”

Section: Anderson Fabry Disease (Afd)mentioning

confidence: 99%

“…LGE within the basal infero-lateral wall without affecting the endocardium is characteristic for a cardiac manifestation in AFD [68]. In genetically confirmed AFD, Moon et al demonstrated that LGE of the infero-lateral wall was present in 50 % of the patients [68]. It also seems that the presence of LGE may have a prognostic value.…”

Section: Anderson Fabry Disease (Afd)mentioning

confidence: 99%

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T1, T2 Mapping and Extracellular Volume Fraction (ECV): Application, Value and Further Perspectives in Myocardial Inflammation and Cardiomyopathies

Roller¹,

Harth²,

Schneider³

et al. 2015

Fortschr Röntgenstr

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!Cardiac magnetic resonance imaging (CMRI) is a versatile diagnostic tool. One of its main advantages is the possibility of tissue characterization. T1-weighted images for scar and T2-weighted images for edema visualization are key methods for tissue characterization. Otherwise these sequences are strongly limited for the detection of diffuse myocardial pathologies. Recently, rapid technical innovations have generated new techniques. T1, T2 mapping and evaluation of the extracellular volume fraction (ECV) allow quantification of diffuse myocardial pathologies and showed great potential in the visualization of fibrosis, edema, amyloid, iron overload and lipid. In the future these techniques might enable the detection of early cardiac involvement, even act as a prognosticator. Moreover, therapy monitoring and follow-up might be possible due to versatile parameter quantification with these new techniques. Key points:▶ CMR allows for tissue characterization via T1-and T2-weighted sequences.▶ In cases of diffuse, global myocardial pathologies, correct image interpretation with traditional CMR sequences might be difficult.▶ T1, T2 mapping and ECV can quantify diffuse, global myocardial pathologies.

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Section: Anderson Fabry Disease (Afd)mentioning

confidence: 99%

Section: Anderson Fabry Disease (Afd)mentioning

confidence: 99%

T1, T2 Mapping and Extracellular Volume Fraction (ECV): Application, Value and Further Perspectives in Myocardial Inflammation and Cardiomyopathies

Roller¹,

Harth²,

Schneider³

et al. 2015

Fortschr Röntgenstr

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“…In general, effects on the heart are well described as a concentric hypertrophic cardiomyopathy with reduced regional myocardial function (Breunig et al 2003;Weidemann et al 2010;Linhart et al 2000). Another key feature is myocardial replacement fibrosis, detected with cardiac magnetic resonance imaging (cMRI) (Moon et al 2003(Moon et al , 2006. In early reports, a short P-R interval was described as one of the typical alterations on electrocardiography (ECG) (Roudebush et al 1973), but a large crosssectional study failed to confirm this finding (Niemann et al 2013).…”

Section: Introductionmentioning

confidence: 99%

Electrical Changes in Resting, Exercise, and Holter Electrocardiography in Fabry Cardiomyopathy

et al. 2015

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Background: In Fabry cardiomyopathy, little is known about the interaction between its key feature of myocardial replacement fibrosis and changes in resting, Holter, and exercise electrocardiography (ÀECG).Methods and Results: Resting ECG, 24-h Holter ECG, and exercise ECG were performed in 95 patients (50 women) with Fabry disease, staged using cardiac magnetic resonance imaging to measure left ventricular fibrosis. With resting ECG, T alterations were seen in patients with cardiac fibrosis, while time intervals and rhythm were unchanged (except for a longer QRS duration in patients with severe fibrosis). All patients with severe fibrosis showed T inversion, ST alteration, or both. With Holter ECG, maximum and minimum heart rate did not differ with fibrosis severity. Patients without fibrotic tissue showed less ventricular premature beats (VPB) (median 5/24 h) compared to those with mild (median 11/24 h) or severe fibrosis (median 115/24 h; P < 0.05, respectively). Fibrosis was a strong predictor of VPB burden (r 2 ¼ 0.5; P < 0.001). During exercise, patients with severe fibrosis had the least increase in systolic blood pressure (sBP) (47 AE 22 mmHg vs. 62 AE 25 mmHg, P < 0.05) and the lowest maximum heart rate (113 AE 18/min; P < 0.05). Patients with mild fibrosis had a high sBP during exercise (198 AE 37 mmHg; P < 0.05). Decreased diastolic blood pressure (>10 mmHg) occurred in some patients with no (3/41) or mild fibrosis (3/ 34) but not in patients with severe fibrosis (0/20; P < 0.01).Conclusions: Our data suggest that cardiac replacement fibrosis is responsible for repolarization abnormalities on resting ECG and increased VPB with Holter ECG. During exercise ECG, advanced cardiomyopathy is characterized by chronotropic incompetence with limitations on blood pressure and heart rate increase.

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“…Myocardial involvement in Anderson-Fabry disease leads to myocardial fibrosis, which can be visualized by late enhancement CMR imaging (129,130). The amount of fibrotic tissue as defined by late enhancement imaging correlates well with the extent of LV hypertrophy, and it is more frequently observed in the inferior lateral wall.…”

Section: Other Nonischemic Cardiomyopathiesmentioning

confidence: 99%

The emerging clinical role of cardiovascular magnetic resonance imaging

Kumar¹,

Patton²,

Friedrich³

2010

Canadian Journal of Cardiology

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Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease Evidence for a disease specific abnormality of the myocardial interstitium

Abstract: These observations suggests that myocardial fibrosis occurs in AFD and may contribute to the hypertrophy and the natural history of the disease.

Cited by 415 publications

References 21 publications

T1, T2 Mapping and Extracellular Volume Fraction (ECV): Application, Value and Further Perspectives in Myocardial Inflammation and Cardiomyopathies

T1, T2 Mapping and Extracellular Volume Fraction (ECV): Application, Value and Further Perspectives in Myocardial Inflammation and Cardiomyopathies

Electrical Changes in Resting, Exercise, and Holter Electrocardiography in Fabry Cardiomyopathy

The emerging clinical role of cardiovascular magnetic resonance imaging

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